What is the management and treatment for a patient diagnosed with Steven Johnson's (Toxic Epidermal Necrolysis) syndrome?

Management and Treatment of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis

Patients with Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) with >10% body surface area epidermal loss should be admitted without delay to a burn center or intensive care unit with experience in treating SJS/TEN and facilities to manage extensive skin loss wound care. 1

Initial Assessment and Triage

1. Severity Assessment:

   • Calculate SCORTEN within first 24 hours to predict mortality risk 1, 2
   • Document extent of skin involvement (% body surface area affected)
   • Higher SCORTEN scores correlate with increased mortality (score of 3 = 32% mortality)

2. Immediate Actions:

   • Stop the culprit drug immediately
   • Transfer to specialized center with SJS/TEN experience
   • Establish multidisciplinary team approach

Supportive Care

Environment and Positioning

• Barrier-nurse patient in a side room controlled for humidity
• Use pressure-relieving mattress
• Maintain ambient temperature between 25-28°C 1
• Handle skin carefully to minimize shearing forces

Skin Management

• Conservative Approach (recommended for all patients) 1:

  • Cleanse wounds gently using warmed sterile water, saline, or dilute chlorhexidine (1/5000)
  • Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over entire epidermis including denuded areas
  • Leave detached epidermis in situ as biological dressing
  • Decompress blisters by piercing and expression or aspiration of fluid
  • Apply non-adherent dressings to denuded dermis (e.g., Mepitel or Telfa)
  • Use secondary foam or burn dressing to collect exudate

• Surgical Approach (consider for TEN with >30% BSA involvement if conservative management fails) 1:

  • Remove necrotic/loose infected epidermis
  • Clean wounds using topical antimicrobial agent
  • Consider debridement with specialized equipment
  • Apply biological or synthetic skin substitutes

Infection Control

• Take swabs for bacterial and candidal culture from lesional skin every other day
• Administer systemic antibiotics only if clinical signs of infection present 1
• Avoid prophylactic antibiotics as they may increase skin colonization with Candida

Fluid Management

• Site venous lines through non-lesional skin when possible
• Monitor fluid balance carefully; catheterize if necessary
• Establish adequate IV fluid replacement initially
• Fluid requirements are lower than predicted by burn formulas 1, 2
• Avoid overaggressive fluid resuscitation 2

Nutrition

• Provide continuous enteral nutrition throughout acute phase
• Deliver 20-25 kcal/kg daily during early catabolic phase
• Increase to 25-30 kcal/kg daily during recovery phase 1
• Use nasogastric feeding if oral intake is precluded by buccal mucositis

Pain Management

• Use validated pain assessment tools at least once daily
• Provide adequate analgesia for comfort at rest
• Add supplementary opiates as required 1

Specific Treatments

Pharmacological Interventions

• Cyclosporine: 3 mg/kg daily for 7-14 days shows benefit in reducing mortality 1, 2
• Corticosteroids: May be considered for severe cases, though evidence is mixed 3, 4
  • Prednisone is FDA-approved for severe erythema multiforme (Stevens-Johnson syndrome) 3
  • If used, administer within 72 hours and taper rapidly within 7-10 days 4
• IVIG: Consider for severe cases, though evidence of benefit is inconsistent 5, 6

Mucosal Care

Ocular Management

• Ophthalmology consultation within first 24 hours
• Regular eye examination and cleaning
• Apply lubricants and topical antibiotics as directed by ophthalmologist
• Schedule follow-up with ophthalmologist within weeks of discharge 1

Oral Care

• Regular oral hygiene
• Topical anesthetics for pain relief
• Antiseptic mouthwashes

Genital Care

• Daily cleansing
• Apply emollients
• Consider gynecological/urological consultation to prevent adhesions

Discharge and Follow-up

1. Patient Education:

   • Provide written information about drugs to avoid
   • Encourage wearing MedicAlert bracelet 1
   • Report to pharmacovigilance authorities (Yellow Card Scheme in UK)

2. Follow-up Appointments:

   • Dermatology or burns/plastic surgery within weeks of discharge
   • Ophthalmology if eye involvement occurred
   • Monitor for complications in multiple organ systems 1

3. Psychological Support:

   • Consider psychological evaluation
   • Refer to support groups (e.g., SJS Awareness)

Common Pitfalls and Caveats

• Delayed recognition and transfer: Early transfer to specialized unit is associated with improved survival 1, 5
• Inappropriate fluid management: Avoid overaggressive fluid resuscitation which can lead to pulmonary edema 2
• Prophylactic antibiotics: May increase skin colonization with resistant organisms 1
• Inadequate pain control: SJS/TEN is extremely painful; regular assessment and adequate analgesia are essential
• Overlooking ocular involvement: Can lead to permanent visual impairment; early ophthalmology consultation is crucial
• Failure to identify and discontinue culprit drug: Immediate withdrawal is mandatory for improved outcomes 6, 7

SJS/TEN is a true dermatological emergency with high mortality rates. A multidisciplinary approach with early transfer to a specialized unit, meticulous supportive care, and careful monitoring for complications is essential for improving outcomes.